Pulmonary Hypertension

Expectations from the progression of PH

03/08/2008

Question:

My mother has severe pulmonary hypertension. I have read a great deal about this disease but nothing is mentioned about what we can expect as she worsens. Will she have a stroke, a heart attack or will she just stop breathing? my question is what usually happens medically as the disease progresses to death? Thank you.

Answer:

Thank you for your question. In patients with severe pulmonary hypertension, the most common cause of death is progressive right ventricular failure - in essence, heart failure. A smaller number of patients die suddenly, potentially from heart rhythm abnormalities and another small group die of intervening conditions such as pneumonia.

In practical terms, patients develop progressive difficulty breathing with exertion and eventually they become short of breath while at rest. They frequently develop swelling of the legs and/or abdomen which does not respond well to medication. If the patient is not a candidate for further therapy such as lung transplantation, they frequently benefit from palliative care, often provided by hospice, to ensure that they are comfortable during the advanced stages of their illness.

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